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Worldwide buy cialis professional 20mg without a prescription erectile dysfunction protocol pdf free, tuberculous pericarditis is the most common cause of constrictive pericarditis (43) buy genuine cialis professional line erectile dysfunction pump nhs. Herniation of the apical left ventricular wall is more subtle in this four-chamber view in systole (A) buy cialis professional 20 mg with amex erectile dysfunction doctors in maine, the apical herniation (arrow) becomes more apparent in the left ventricular outflow tract view in late diastole (B). Early diastolic filling will be normal, with limited mid- and late-diastolic filling. Pulmonary wedge and central venous pressures are increased due to elevated ventricular filling pressures (110). Hepatomegaly, splenomegaly, jugular venous distension, edema, or ascites may occur. Auscultation reveals a diastolic filling sound corresponding to abrupt cessation of ventricular filling (precordial knock) (108,111). Chest radiography may be normal or may display macroscopic pericardial calcification in 25% of patients (see Fig. The superior and inferior vena cavae will be dilated due to elevated ventricular filling and central venous pressures. Subcostal imaging may demonstrate “diaphragmatic tethering,” where the diaphragm is pulled toward the heart with each ventricular contraction. Doppler echocardiography shows marked respiratory variation of both left- and right-sided inflows (Fig. With inspiration, there is an exaggerated decrease in the mitral inflow velocity (mitral E velocity) and an exaggerated increase in tricuspid inflow velocity (tricuspid E velocity) (112). Conversely, in expiration, there is an exaggerated increase in mitral inflow velocity and an exaggerated decrease in tricuspid inflow velocity. These diagrams illustrate a patient with constrictive pericarditis and the corresponding Doppler echocardiographic patterns with inspiration and expiration. Inspiration starts with the upward deflection of the respirometer tracing, while expiration starts with the downward deflection of the tracing. Note the decrease in mitral inflow E velocity with inspiration, and increase with the onset of expiration (left frame). Cardiac catheterization demonstrates equalization of left and right ventricular end-diastolic pressures, left and right mean atrial pressures, and the mean pulmonary capillary wedge pressure. The “square root sign” refers to the early diastolic pressure decrease followed by a plateau on left and right ventricular pressure tracings, and results from rapid early diastolic filling with abrupt cessation (see Fig. The definitive treatment for constrictive pericarditis is radical pericardiectomy (99,111). Differentiating Constrictive Pericarditis from Restrictive Cardiomyopathy Restrictive cardiomyopathy (see Chapter 56) is an infiltrative process, and includes amyloidosis, hemochromatosis, endomyocardial fibrosis, and eosinophilic cardiomyopathy. It also may be idiopathic (113,114) and is characterized by markedly abnormal diastolic function with preserved systolic function. The differentiation between constrictive pericarditis and restrictive cardiomyopathy often is difficult (110,115,116,117). Echocardiographic measurements of diastolic function in children are confounded by factors including preload, heart rate, age, and body size (118). Differentiating between constriction and restriction is critical, since the definitive treatments for these disorders are markedly disparate (pericardiectomy vs. Cardiac catheterization can be useful in differentiating constriction from restriction (Fig. This study evaluated adult patients and requires validation in a pediatric cohort. Echocardiographic differentiation between constriction and restriction includes the factors listed in Table 61. Mitral inflow, hepatic venous flow, tricuspid inflow, and pulmonary venous inflow velocities are affected by respiration in constriction. There may be a relatively normal peak mitral valve E velocity during expiration in constriction or restriction. Normally, hepatic vein Doppler waveforms will demonstrate larger systolic and diastolic forward flow and smaller systolic and diastolic flow reversal waves. In constriction, decreased left ventricular filling with inspiration allows for increased right ventricular filling, causing hepatic vein diastolic forward flow to increase. In expiration, hepatic vein diastolic forward flow decreases, and significant diastolic flow reversal occurs. Conversely, in restriction, marked reversals in the hepatic veins occur with inspiration in both systole and diastole. Mitral inflow, tricuspid inflow, and pulmonary vein velocities rarely are affected by respiration in patients with pure restriction. Importantly, the diastolic flow reversals seen on expiration in constriction may not be evident in patients with tachycardia or atrial fibrillation. In these situations, augmented systolic reversals actually may be seen with expiration. Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory. Note the relatively similar peak mitral valve E velocity in normal, constriction, and restriction in expiration. In patients with constriction, marked diastolic reversals will be seen with expiration (arrow), while the flow may appear normal with inspiration. Conversely, in restriction, marked reversals in the hepatic veins are typically seen with inspiration, and may occur in both systole and diastole (arrows). Note the marked decrease in tissue Doppler early diastolic mitral annulus (e′) velocities in patients with restrictive cardiomyopathy (typically below 8 cm/s), while patients with constrictive pericarditis have normal or increased e′ velocities. In normal children beyond infancy, the early diastolic septal mitral annulus velocity (e′) should be between 9 and 16 cm/s. In restriction, the septal e′ velocity often is less than 8 cm/s (similar to other cardiomyopathies) (15,119). In normal hearts, the lateral mitral annulus e′ velocity is greater than the septal mitral annulus e′ velocity. In constriction, the septal mitral annulus e′ velocity can be greater than or equal to the lateral mitral annulus e′ velocity, a paradoxical finding called mitral annulus reversus (120). This reversal of mitral annulus velocities is not seen in patients with restrictive cardiomyopathy. In their study, they found that: (1) respiratory-related ventricular septal shift, (2) tissue Doppler medial e′ velocity ≥9 cm/s, and (3) hepatic vein expiratory diastolic reversal ratio ≥0. Using these “Mayo Clinic Criteria,” a combination of septal shift with either of the other two criteria gave the highest sensitivity (87%) and specificity (91%) for diagnosis of constrictive pericarditis (121). Special Circumstances Patients during Mechanical Ventilation During normal breathing, there is a decrease in intrathoracic pressure with inspiration and an increase with expiration. During positive pressure mechanical ventilation, the intrathoracic pressure changes are opposite those that occur with spontaneous breathing. Mechanical inflation of the lungs causes an increase in intrathoracic pressure (122).

Mutations in these proteins result in plasma membrane damage in striated muscle as seen in muscular dystrophy–associated cardiomyopathies cheap cialis professional line erectile dysfunction doctors in st louis mo. Loss of normal dystrophin function in the heart produces four-chamber dilation purchase generic cialis professional on line erectile dysfunction suction pump, reduction in left ventricular function buy discount cialis professional 40 mg line erectile dysfunction drugs staxyn, and arrhythmias (69). Since dystrophin complex–related cardiomyopathies will be covered in great detail in a later chapter, this section will be restricted to an overview of dystrophin complex organization and function, as well as functional classes of mutations. Dystrophin is a rigid cytoplasmic protein that anchors dystroglycan to the cytoskeletal, filamentous actin (70). Removal of dystrophin from the dystrophin glycoprotein complex makes this complex unstable and leads to its loss from the plasma membrane, rendering the cell susceptible to damage from contraction (71). Specifically, the 2+ 2+ loss of membrane integrity allows Ca entry into the cell, which in turn activates Ca -sensitive proteases, leading to cellular degradation and release of cardiac myocyte proteins such as creatine kinase into the bloodstream (72,73). In cardiac myocytes, mutations in dystrophin also affect the function of stretch-activated ion channels, which normally open in response to stretch during ventricular filling (72). Heterodimeric dystroglycan is a protein central to the dystrophin glycoprotein complex that spans the sarcolemma and binds to laminin in the surrounding basal lamina through its α-dystroglycan subunit and to dystrophin through the cytoplasmic, carboxy-terminus of its β-dystroglycan subunit (74,75). Sarcoglycans are transmembrane glycoprotein complexes comprising six isoforms (α, β, δ, ε, γ, ζ) that are thought to stabilize the interactions between α and β dystroglycans. Dystrobrevin binds to dystrophin and the sarcoglyan complexes and also plays an important role as a structural scaffold linking the dystrophin glycoprotein complex to intermediate filaments (76). Mutations in dystrobrevin have been associated with left ventricular noncompaction, a cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep trabecular recesses (77). These linkages cluster to form focal adhesions and provide a route through which external physical forces are transduced into intracellular process. This has important functional implications since increasing matrix stiffness changes cardiomyocyte force production. Cardiomyocytes exerted lower contractile force on substrates at stiffness values higher than the native myocardium (82). This provides evidence that substrate stiffness may play a crucial role in the proper development of the heart. To summarize these observations, the mechanical environment is important to the balance of intracellular forces and phenotype of fibroblasts, regulating matrix degradation, synthesis, and modification. Increased stiffness causes an increase in integrin receptors and changes in cytoskeleton such as actin organization and increases in filamentous F-actin relative to globular G-actin. Cell–Cell Interactions Intercalated discs are highly organized and specialized components of the cardiac myocyte that maintain structural integrity and synchronized contraction of cardiac tissue (Fig. Intercalated discs are located at the longitudinal ends of the rod-shaped cardiac myocytes where contact and cell–cell communication occurs, and are composed of three different types of connections: adherens junctions, desmosomes, and gap junctions. Adherens junctions and desmosomes provide mechanical coupling for force transmission and reinforce cardiac myocyte structure, whereas gap junctions are essential for rapid electrical transmission between cells. Components of adherens junction include the transmembrane cadherin (N-cadherin) responsible for cell–cell adhesion, the cytoplasmic catenins (α-β-γ [plakoglobin]) that bind to cadherin and regulate adhesion and other catenin-related proteins including vinculin and α-actinin which link the intercalated discs to the cytoskeleton catenins (84). Adherens junctions hold cells tightly together as the heart expands and contracts and act as the anchor-point where myofibrils are attached, enabling transmission of contractile forces from one cell to another (85). Because actin filaments also pull against cadherins, these junctions also mediate the bidirectional transmission of cytoskeletal tension between cells (63,86). The desmosomal cadherins desmocollin and desmoglein interact in a heterophilic manner in the extracellular space to connect adjacent cells. The cytoplasmic component of desmosomes consists of the proteins plakoglobin (γ-catenin), plakophilin, and desmoplakin, the latter of which connects the desmosome to intermediate filaments such as desmin (85). Thus, desmin is uniquely situated to integrate signals from both cell–cell and cell–matrix interactions to ensure cellular integrity, force transmission, and biochemical signaling (35). Given this crucial role, it is not surprising that mutations in desmin lead to cardiomyopathy (87). The gap junctions maintain electrical coupling of individual myocytes to form an electrical syncytium. Gap junctions ensure the proper propagation of the electrical impulse, which triggers sequential and coordinated contraction of the myocardium. These isoforms also exhibit distinct regional, cell type–specific and chamber-specific expression, with different isoforms present in the conduction system as compared to the ventricular myocardium (90). Six connexins combine to form one connexon that extends from the plasma membrane of one cell to dock with a connexin of an adjacent cell, creating an intercellular gap (88). B: Expression of Cx43 (green) and α-actinin (red) at different stages of human cardiac development. Cx43 progressively relocalizes from the myocyte lateral membrane toward the intercalated disc (Upper left, 10. Arrows indicates less intense staining in the intercalated disc at the age of 5 years compared to the intensity of lateral signals. Assembly of the cardiac intercalated disc during pre- and postnatal development of the human heart. Gap junction channel assembly, membrane localization, gating, and degradation are regulated by a variety of stimuli including voltage, ionic concentrations, pH, phosphorylation, and local protein interactions. During cardiac myocyte development and maturation, large changes in the spatiotemporal distribution of gap junctions, desmosomes, and adherens junctions occur. In the mature myocardium, all three are clustered in a bipolar pattern (perpendicular to the long axis) on the ends of the myocyte. However, during embryologic development, adherens junctions are also found on the lateral membranes where they seem to be able to sense mechanical forces along the transverse axis and are thought to play an important role in myofibrillogenesis (63,93). At the perinatal stage, the adherens junctions no longer surround the entire cell, but are restricted to intercalated discs between cells. Interestingly, this polarization coincides temporally with an increase in cardiac output at birth to support the needs of the newborn, suggesting that maturation of contractility provides mechanical inputs for cadherin movement to the longitudinal border (63). Most of the adherens junction proteins were completely localized to the intercalated disc by 12 months after birth (Fig. In contrast, there was sparse, diffuse connexin-43 expression in fetal hearts that gradually increased after birth but does not fully segregate to the intercalated disc until 7 years of age (94). The functional implications of these differences are unclear, but may partially explain the ability of neonatal cardiac myocytes to propagate electrical impulses in both the longitudinal and perpendicular axes (“isotropic”), compared to the “anisotropic” adult myocytes that predominantly exhibit longitudinal impulse conduction (94,95). Several cardiac disorders have recently been identified in which defective electromechanical coupling between cardiac myocytes leads to degenerative cardiomyopathies characterized by contractile impairment and electrical disorders. Mutations in proteins in the adherens junctions are associated with heart failure and dilated cardiomyopathy (84). Coronary Vasculature The spontaneously contracting heart tube is initially formed as an avascular organ. The cells that form the tissues of the coronary system move onto the surface of heart after the looping stage of cardiogenesis, making first contact at the future site of the atrioventricular septum. The specific origins of the coronary endothelial cells have been the rigorously debated; as a number of different approaches for determining their origins have resulted in conflicting conclusions (99,100,101). Regardless of the cellular origins, the signals that regulate coronary development are derived from both the epicardium and cardiac myocytes (99). Both metabolic (hypoxia) and mechanical factors stimulate growth factors that promote angiogenesis (102). The coronary vessels begin to coalesce from mesenchymal cells via vasculogenic processes in the extracellular matrix-rich, subepicardial space between the epicardium and the myocardium (105).

These professionals ensure that instrumentation is well maintained cialis professional 20 mg on line erectile dysfunction joliet, ready for use on an emergency basis order cialis professional uk erectile dysfunction scrotum pump, and quickly repaired if necessary discount cialis professional 40mg with mastercard erectile dysfunction injection dosage. However, it may be a unique experience for the infant and parents in the outpatient setting. The clinical state of infants may vary: they may be content and comfortable, difficult to console, irritable, excessively sleepy or lethargic, or comatose. The technologist must consider the state of each infant and determine the best method to make the infant as comfortable as possible to obtain a complete recording. This may require having the infant fed, having diapers changed, adjusting room temperature and, often, just prolonging the recording until the infant becomes comforted. The purpose of these efforts is to facilitate a recording of spontaneous sleep cycles as well as an awake portion. Typically, nine scalp positions are used (Fl, F2, C3, C4, Cz, T3, T4, O1, O2), although others may be added. In addition, electrodes are placed at Al and A2, and a ground electrode is placed either at mid-forehead or on a mastoid region. Because digital recordings are fundamentally referential, an additional reference electrode position may be needed (typically noncephalic), although some instruments provide a so-called “internal” reference. The Fl and F2 electrode positions are 20% of the inion-nasion distance above the nasion and 10% of the circumferential measurement from the midline. Electrode placement for the neonatal electroencephalogram designated by bolded circles. After head measurement, the scalp is prepared with slight abrasion at each electrode site with a mild abrasive gel, using the soft end of a cotton applicator. A ball of cotton is placed over each electrode, and the electrode array is finally secured with paper tape. The environment in which collodion is applied must be well ventilated because it is flammable and may be toxic to the lungs. This is a particular problem for infants in special care units who may be in confined areas such as isolettes that may concentrate fumes and for those infants with already compromised pulmonary function. The same procedures are followed for electrode application as described earlier for scalp electrodes. This assists in staging sleep and in the determination of the origin of some electrical potentials recorded in anterior cephalic electrodes that may have been generated by eye movement. In addition, limb movements may be detected and characterized by triaxial accelerometry (Frost et al. These are most often used in research protocols or special clinical circumstances. Intraarterial blood pressure can be measured from an indwelling catheter already placed for clinical P. These are best achieved in a sustained recording by using a single, bipolar montage with broad coverage over the scalp. In the recording of older children and adults, localization of focal abnormalities often requires the use of several montages; however, because of the range of abnormalities in neonates and the overriding need to characterize state changes over time, multiple montages are not used. A typical montage, with adequate coverage over the scalp, including the required channels with a Cz electrode placement, is given in Table 2-2. New data obtained from these recordings may result in additional guidelines for neonatal recordings. Paper speed is set at 30 mm/sec for analog recordings or 10 sec/screen or “page” for digital recordings. These paper-speed settings are used in many laboratories in the United States and are used in this atlas. However, several laboratories, particularly those with ties to the French school of recording, use a slow speed: 15 mm/sec or 20 sec/screen or “page. However, many of the difficulties that were present during the development of these techniques still persist. Instrumentation, camera mount, electrode cables, and junction box must all be placed not to interfere with the ongoing care of the neonate. It also is important to keep the video image as free as possible from personnel and instruments that block the camera view. In addition, during paroxysmal clinical events, it is essential for personnel not to interfere with the recording of the entire seizure. The ambient temperature of the recording environment should be controlled as well as possible. However, for infants with suspected seizures, it is essential that the infants remain uncovered, with all limbs in full view of the camera. These concerns must be addressed with nursing staff at the onset of the recording session at the bedside and with parents. Infant Positioning Infants may be positioned by nursing or attending physician staff for clinical purposes. A number of constraints occur in maintaining a good video image: Limbs with intravascular line placements may be restrained; agitated infants may be swaddled; some infants may be intubated with limited range of head and neck movement; wound dressings may be present; or infants must remain covered because of temperature instability. These and other problems must be addressed to obtain the best video image for each study. In addition, the camera must be mounted so that it is directly above the neonate to provide a full view of all limbs and head without the distortion that comes from a camera placed at the foot or to the side of the infant. Lighting Lighting in neonatal special care areas can be suboptimal for video recording. Ambient light is dictated by clinical needs and day/night cycles imposed for optimal infant adaptation. When additional light is used to enhance picture quality, additional problems may arise. Added light may provide added heat and cause the infant to perspire, enhancing sweat artifact. These problems must be considered before beginning video recordings and must be corrected when encountered during monitoring. Potential for Missed Events A potential is always present for missing the video recording of important paroxysmal clinical events. To maximize video-recording yield, the technologist must first arrange the video camera and begin video recording. It is terminated only after all electrodes are removed, the infant is cleaned, and the instrument is removed. This can be based on a basic algorithm adaptable to individual recording circumstances (Fig. The record should be annotated when behavioral or autonomic changes occur or when other events happen that may affect the record. It is important that the technologist and clinical neurophysiologist know what behaviors suspected by the referring physician are thought to be clinical seizures. In addition, if the type of paroxysmal behavior for which the infant was referred occurs during the recording, the event should be noted and described. Other abnormal behaviors also should be noted on the record at their time of occurrence.

The disease is not always circumferential cheap cialis professional 20 mg without prescription erectile dysfunction drugs from canada; one of the features that distinguish it from ulcerative colitis is that it may involve only one portion of the circumference of the bowel cheap cialis professional 40mg free shipping erectile dysfunction organic causes. Another important diagnostic feature is the presence of the so-called ‘skip lesion’ (Fig cheap cialis professional 40 mg on line erectile dysfunction when cheating. Skip lesions are virtually ‘cobblestone’ appearance due to criss-crossing fne ulceration. However, the entire colon may be involved or the disease may be limited to just one Diverticular disease segment. The rectum is often spared – another important Diverticula are sac-like out-pouchings of mucosa through differentiating feature from ulcerative colitis. Very deep ulcers are present; two examples of an ulcer tracking in the submucosa are arrowed. A long stricture is present in the transverse colon (between the curved arrows) and a shorter one in the sigmoid colon (between the small arrows). These two abnormal segments with normal intervening bowel are an example of ‘skip lesions’ – an important diagnostic feature of Crohn’s disease. The term diverticulitis is used when infection of the affected segment causes symptoms such as sepsis, diarrhoea or obstruction. The diverticula, when flled with barium, are seen as spherical out-pouchings with a narrow neck (see Fig. Numerous diverticula are seen as of the stricture, it is impossible to defnitely exclude a carcinoma. An appendicolith may be visible stranding of the surrounding fat due to oedema and infam- within the appendix as a hyperechoic area casting an acous- matory change (Fig. An appendix abscess can be diagnosed abscess or fstula into the bladder, small bowel or vagina. Occasionally, diverticula perforate directly into the peritoneal cavity causing peritonitis, and Acute infarction of the large bowel is very rare. Ischaemia free intraperitoneal air should be looked for on a plain is usually a more chronic process giving rise, initially, to abdominal flm if necessary. Usually, Chronic mesenteric ischaemia is often a delayed diagnosis this is clearly within an area of recognizable diverticular as patients may present with vague symptoms, and not the disease. It is, however, often impossible to differentiate classic history of post-prandial pain. It occurs due to nar- such a stricture from a carcinoma occurring coincidentally rowing of the arteries supplying the bowel, and usually at in a patient with diverticular disease. In cases of doubt the and helping determine whether revascularization can be diagnosis can be made with ultrasound, which shows a undertaken by an endovascular or surgical approach. In the distended, non-compressible appendix with a thickened later stages, a stricture may form (Fig. If stricture formation occurs, the stricture Sacculations may be seen arising from one side of the stric- will be smooth and have tapered ends. Gastrointestinal Tract 183 Pneumatosis coli In this unusual condition, gas-flled spaces are present in the wall of the bowel. They can be identifed on a plain flm of the abdomen, but the diagnosis is much easier with a barium enema where the cysts cause smooth, translucent flling defects projecting from the wall of the bowel (Fig. The appearance could be confused with intramural haemorrhage and oedema, or with colitis if the presence of air within the cysts is not appreciated. This happens most frequently in the sigmoid colon, particularly when it is redundant, and less often in the caecum. The twisted loop becomes greatly distended and the bowel proximal to the volvulus is obstructed by the twist and may, therefore, also be dilated. Intussusception An intussusception is the invagination of one segment of the bowel into another. Part of the colon showing numerous By far the commonest type is an ileocolic intussusception, translucencies in the colon wall owing to many gas-flled cysts. Other types are colocolic, when the colon invaginates into another part of the colon, and ileo-ileal when the ileum invaginates into a per rectum under fuoroscopic or ultrasound control, the more distal segment of ileum. In infants and a reduction is to be safely carried out, the child should have young children, in particular, the diagnosis is often con- no clinical signs of peritonitis. The longer the symptoms frmed by an enema with air or carbon dioxide as the have been present, the greater the risk of perforating gan- contrast agent, and an attempt at reduction of the intus- grenous bowel. When gas is insuffated presence of a neoplasm, typically in the submucosa, such 184 Chapter 6 Fig. A sausage-shaped mass less than 1 cm in size, and very few less than 2 cm, are is demonstrated, which has mesenteric fat within the cancers. The features that suggest malignancy are a diam- lumen of the intussuscipiens (Fig. Surgical treatment eter of more than 2 cm, a short thick stalk or an irregular is invariable. They are, there- Polyps fore, removed endoscopically when discovered, regardless The word ‘polyp’ means a small mass of tissue arising from of whether or not an individual polyp is believed to be the wall of the bowel projecting into the lumen. They may be single or multiple and on radiological grounds to exclude frank malignancy in a are found most frequently in the rectosigmoid region. Gastrointestinal Tract 187 familial polyposis they are numerous and one or more will, these two sites are usually quite different. They are usually large when frst dis- become very large without obstructing the bowel, so covered and are frequently mistaken for faeces. The anaemia and weight loss are the common presenting common sites are the rectum and caecum. Almost all isolated polyps in children are strictures are rarely more than 6 cm in length. Multiple primary tumours must be excluded, as a patient • Hyperplastic or metaplastic polyps. The appearance and behaviour of a carcinoma in barium enema for the diagnosis of colonic carcinoma. The mesorectal fascia is an suspected recurrent disease in patients who are to be con- important anatomical landmark as it represents the surgical sidered for potential curative surgery (e. The dis- for liver metastases) to ensure there are no other sites of tance between tumour invading the mesorectal fat and the recurrence. The possibility of an involved Hirschsprung’s disease (congenital aganglionosis) surgical margin can be accurately anticipated (Fig. Hirschsprung’s disease is due to the absence of ganglion The role of endoscopic ultrasound in rectal cancer is limited cells beyond a certain level in the colon, usually in the to very early stage disease as some tumours cannot be sigmoid or rectosigmoid region. Note the benign hyperplasia of the prostate (P) and a slightly trabeculated bladder (B). Note the mesorectal fascia (black arrows) that encases the mesorectal fat and the rectum. Note the transition between the normal calibre aganglionic rectum and the dilated sigmoid colon. The aganglionic segment, usually the rectum, is either Anal fstula and perianal abscess normal or small at barium enema and the diagnosis depends on recognizing the transition from the normal or Most anal fstulae are simple low tracks that can be assessed reduced calibre colon to the dilated colon (Fig.